Dementia Aware  

Young onset dementia

What you need to know about younger people with dementia (YOD)

Young onset dementia, also known as early onset dementia, describes any neurocognitive disorder (dementia) diagnosed in someone under 65.

Due to the population increase from the Baby Boomer generation, young people developing young onset dementia is becoming more common, with approximately one person in every 1,000 below 65 developing young onset dementia (Draper and Withall, 2016).

In 2016, approximately 200,000 people were diagnosed with young onset dementia in the United States, 42,325 in the United Kingdom, 25,938 in Australia, and 16,000 in Canada (Alzheimer Society, 2016).

While each person’s experience with dementia is different and although the symptoms of dementia are similar regardless of age, younger people with dementia have many different needs and challenges compared to the older person with dementia.

The most common types of young onset dementia include:

  • Familial Alzheimer’s disease (30 per cent)
  • Vascular dementia (18 per cent)
  • Frontotemporal dementia (12 per cent)
  • Alcohol related dementia (10 per cent)
  • Lewy Body dementia (10 per cent)
  • Rare and unusual dementia (20 per cent)
         • Down’s syndrome dementia
         • Creutzfeldt Jakob disease(CJD)
         • HIV-neurosyphilis
         • Corticobasal degeneration

Young onset dementia can be difficult to recognize and can mimic other illnesses — depression, anxiety, stress related illnesses, peri-menopause.

It is extremely important that any person suspected of having early onset dementia has a full assessment: cognitive and neurological assessment, blood work, and CT scan of head.

Some of the early signs are:

  • Memory loss
  • Easily stressed, frustrated or angered
  • Behaviour and/or personality change “does not seem like him/herself”
  • Changes in walking, balance, coordination
  • Subtle changes in communication — forgetting words, mixes up words

For the young person, the diagnosis of dementia is much harder to accept as it is completely unexpected; it is a life-changing moment for everyone in the family unit.

The person is usually in the prime of life, employed, supporting and caring for a family, socially outgoing, physically active and healthy, he/she may also be caring for older parents.

Now, the person, the entire family must adapt to a different way of life, to the changing circumstances, and to the changing roles and responsibilities.


The emotional impact upon hearing the diagnosis will be different for each person, and can range from disbelief, denial, frustration, anger, sadness, and depression. All these emotions are perfectly normal and to be expected.

Consider going to family counselling, the opportunity to share feelings and fears will help everyone as they try to understand the diagnosis, If the dementia is genetic, then counselling/testing may be necessary.

You may want to share the diagnosis with friends; it is your decision, but is a good idea for you to have a support network of people you trust who can help support you and your family.


It important to remember you can still do the things you enjoy and live a full life.

Yes, it will be different, you will likely have to make some changes; however, living well means adapting to changes as they occur. Continue to meet with friends, go out for dinner, hockey games, etc.

Unfortunately, many community programs for dementia support the older/retired person and may not be a good fit, so contact the Alzheimer Society of B.C. or Interior Health for information about resources and supports.

Financial and Legal Planning

The sooner you make decisions about your health care, your finances and your estate, the more in control you will feel and the better prepared you are for the future and ensuring your family are taken care of.

You should have:

  • Will
  • Filed income tax
  • Enduring Power of Attorney (financial/legal affairs) *
  • Representation Agreement (personal care/health care decisions) *
  • Advance Directive which is a written summary of your wishes and instructions for future health care*
  • If you are the ‘Breadwinner’ talk to a Financial Advisor/ Bank re: assets, mortgage, debts
  • For advice re: disability, financial aid, contact Interior Health for Social Worker support

​*For further information on Power of Attorney, Representation Agreements and Advance Care Planning see www.nidus.ca.


You may be able to continue working, however, if your job requires driving, operating machinery, or may affect safety, then your employer must be notified.

Depending on the work you do, some adjustments to work environment and/or work schedule may be necessary. Working will provide income, socialization, a sense of purpose/satisfaction, and a temporary distraction.

Daily Life

  • Follow a healthy diet, exercise, cognitive stimulation exercises
  • Continue to participate in sporting, social activities
  • Focus on your strengths, remain active and involved in family life and community activities
  • Initially, you may continue driving, however, over time, you may find it becomes more difficult and not safe. Talk with your doctor and family. If you wish to continue driving, you will be required to take a road safety test
  • If mobility or balance is worsening, consider using adaptive aids — shower stool, grab bars and request a home safety assessment from Interior Health Community Care
  • To help stay organized and remember appointments, keep a journal or diary, write important events on large calendar on wall or refrigerator, look at assistive technology options e.g. talking clock, medication reminders
  • Write letters, make videotape, compile a memory book for family and friends


www.uwaterloo.ca/ ..../young-onset-dementia-us-us-guide
First Link Dementia Helpline, 1-800-936-6033


Rare, unusual dementias

What you need to know about other types of dementia

Dementia is a common and growing problem worldwide.

While 95 per cent of cases are attributable to Alzheimer’s disease, vascular dementia, Lewy body dementia etc., little is known about the remaining five per cent of dementia cases.

These types of dementia are extensive, and are known as rare and unusual dementias.

In 2002, a European Union project on rarer forms of dementia estimated a prevalence of five cases per 10,000 people in the community (Alzheimer’s Europe 2002-2003).

Rare and unusual dementias are classified into 4 groups:

  • Degenerative Causes: Familial Alzheimer’s disease, progressive supranuclear palsy
  • Vascular Causes: CADASIL, normal pressure hydrocephalus
  • Infectious Causes: neurosyphilis, HIV and AIDS dementia complex
  • Human Prion Disease: fatal familial insomnia, Creutzfeldt-Jakob disease

Degenerative Causes of Dementia
Progressive Supranuclear Palsy:

  • Also called Steele-Richardson-Olszewsky syndrome
  • Occurs around age 60 years
  • Characterised by tauopathy (abnormal levels of tau protein) with nigral (eye movement, motor skills) and corticobasal (movement) degeneration which involves the cerebral cortex (outer layer of neural tissue of the cerebrum), plays a key role in memory, awareness, thought, language, attention and perception
  • Classic symptoms: supranuclear gaze palsy (inability to look in a direction based on area of brain affected), backwards arching and rigidity of neck with gait problems and imbalance (tend to fall backwards)
  • Cognitive symptoms: reduced verbal fluency, inattention and executive dysfunction (planning, abstract thinking, behavioural control)
  • Disease progression is slow with initial retention of insight

Vascular Causes of Dementia
Normal Pressure Hydrocephalus:

  • Pathophysiology is unclear, but there is a possible cerebrovascular role
  • Easily mistaken for Alzheimer’s disease or Parkinson’s disease
  • A build up of CSF (cerebrospinal fluid) in the brain ventricles (hollow fluid filled chambers) caused by an obstruction or absorption is impeded
  • Can be secondary to other conditions e.g. brain hemorrhage, meningitis
  • Alzheimer like symptoms occur because the enlarged brain ventricles press on and damage nearby brain structures that affect memory, walking and bladder control
  • Signs and symptoms known as the classic triad: “wet, wacky, wobbly”
    • urinary urgency, frequency and incontinence
    • cognitive impairment, confusion, prominent memory loss, slowness of thought
    • gait disturbance (like walking on a boat), weakness, short, shuffling, wide steps
  • Diagnosis: CT Scan/MRI of head, presence of the classic triad, thorough case history
  • Treatment: CSF drained by lumbar puncture and/or drain CSF from brain by a shunt
  • Prognosis: if effective, gait will improve almost immediately, and the milder the dementia, the more likely it will improve. However, for those with moderate to advanced dementia, there is unlikely to be a significant improvement

Infectious Causes of Dementia

Syphilis is a sexually transmitted disease caused by a bacterial infection. It is treated with Penicillin. Syphilis has been increasing since the advent of the HIV epidemic. Since 1996, rates of syphilis have increased significantly amongst homosexual men and men/women 55 years and older.


  • Neurosyphilis develops in 25-40 per cent of untreated people (Mehrabian, et al., 2009)
  • Usually presents more than 10-25 years after initial infection
  • It is a slow, progressive destructive infection of the brain and spinal cord
  • Changes in personality, cognition, mood, severe executive dysfunction, can present as a frontal lobe type dementia
  • Psychiatric symptoms include mania, grandiose delusions, paranoia
  • Neurological signs include ataxia (poor coordination), disturbed speech, abnormal pupils, nerve pain, pain in extremities, seizures, paralysis
  • Diagnosis: blood tests – rapid plasma regain (RPR)
  • Treatment: IV Penicillin
  • Prognosis: symptoms may be ‘potentially reversed’ if person responds to treatment

Human Prion Disease

A prion is an infectious particle. When it enters the body, the prion begins folding into an abnormal almost 3D shape and triggers the existing normal forms of protein to change into the prion shape.

In the brain, the prion protein affects the grey matter with subsequent nerve loss, gliosis (scarring) and characteristic spongiform change (small round empty spaces).

There are four types of human prion disease that cause dementia:

Sporadic Creutzfeldt-Jakob disease:

  • Most common form (85 per cent)
  • Occurs after age 50 years
  • Early signs: headaches, weight gain, fatigue and depression
  • Then rapidly progressing global dementia, myoclonus, ataxia, progressive pyramidal, extrapyramidal and cerebellar dysfunctions with gait, vision (leading to blindness) and speech problems
  • Diagnosis: EEG (electroencephalograph) CSF analysis, MRI, tonsil biopsy is negative
  • Medications may be given to reduce the severity of movement disorders and for comfort measures
  • Prognosis: rapidly progressing disease where the person can neither move nor speak (akinetic mutism), death follows in six months to one year

Iatrogenic Creutzfeldt-Jakob disease:

  • Caused by inadvertent transmission of CJD between humans mostly via medical procedures e.g. corneal graft, prion infected surgical instruments
  • Symptoms, investigations, treatment as per sporadic CJD, progresses very rapidly

Variant Creutzfeldt-Jakob disease:

  • Also known as mad cow disease
  • Caused by transmission of prion protein variants via food originating from cattle e.g. beef
  • Psychiatric symptoms: depression, personality changes, irritability, aggression, fleeting delusionNeurological deficits: chorea, ataxia, myoclonus, dysaesthesias and dementia
  • Diagnostic tests: MRI, tonsil biopsy for prion protein is positive
  • Life expectancy usually 12–14 months

Familial Creutzfeldt-Jakob disease:

  • Also called human spongiform encephalopathy
  • Accounts for 10-15 per cent of all CJD cases
  • Age at onset can be as early as age 20, but more common ages 40-50 years
  • Symptoms and treatment as per sporadic CJD
  • As this if familial, genetic testing may identify carriers and early/late onset varieties of this disease, therefore genetic counselling is essential.

Reduce your dementia risk

What you need to know about reducing dementia risk factors

The world has experienced many epidemics, but most were the result of infectious disease — Black Death,  Spanish flu  — they came and then went away.

The dementia epidemic is not an infectious disease, and we cannot afford to sit by and wait for it to disappear, because it will not. We must fight it, and the only way to stop an epidemic is prevention (Korczyn & Vakhapova, 2007).

There is no cure for dementia, no one single cause of dementia, and no specific diagnostic test to determine who is at risk of dementia.

Given this information, our goal is the prevention of dementia. However, a delay in the onset of dementia would also be an effective preventative strategy, since research has indicated that delaying the onset of dementia by just five years would half its prevalence. 

Dementia Risk Factors

Non-Modifiable Risk Factors:

  • Age
  • Gender
  • Genetics
  • Learning disabilities (Down’s syndrome)

Potentially Modifiable Risk Factors:

  • Alcohol: whilst low to moderate alcohol consumption is associated with lower risks of coronary artery disease and ischemic stroke, alcohol intake exceeding current recommended limits increases the risk of dementia — e.g. Wernicke’s Korsakoff
  • Head Injuries: there is increasing evidence of a link between brain injury and dementia. Recent studies show an increased incidence of dementia, notably Chronic Traumatic Encephalopathy (CTE) in people who sustained multiple concussions
  • Physical Inactivity: lack of physical activity has clearly been shown to be a risk factor for cardiovascular disease, and a potential risk for high BP, obesity, and type 2 diabetes
  • Smoking: is a risk factor for atherosclerosis and stroke both of which can result in vascular dementia
  • Mid-life obesity:  an elevated body mass index (BMI) in middle age appears to be an important risk factor for dementia, type 2 diabetes, stroke and heart disease. Therefore, the maintenance of a normal weight is a worthwhile intervention in the prevention of dementia (Gorospe & Dave, 2007)
  • Depression: people with depression have two times the risk of dementia (Barnes & Yaff, 2011)
  • Mid-life hypertension: hypertension is a well recognized risk factor for stroke and multi infarct dementia and is now a suspected risk factor in Alzheimer’s disease
  • Diabetes: hyperglycemia can have a direct toxic effect on neurons causing oxidative stress and accumulation of advanced glycation end products which affect brain tissues. Diabetes may also lead to dementia through ischemic cerebrovascular disease, and can develop in a cluster of vascular risk factors which can constitute metabolic syndrome which is already known to be a predictor of stroke (Yaffe, 2004)
  • Hyper-cholestrolemia: mid-life high cholesterol appears to be a risk factor for dementia, and atherosclerosis is believed to be involved in the development of dementia, notably vascular dementia and Alzheimer’s disease
  • Cognitive inactivity: based on the concept of use it or lose it

The first World Health Organization Ministerial Conference on Global Action Against Dementia (2015), identified the following strategies that can potentially reduce the risk of developing dementia and/or delay the onset of dementia:

Risk Reduction Strategies:

  • Keep physically activity: exercise for 30 minutes, 4-5 times per week.
  • Lose weight: follow a healthy weight loss plan and exercise program as per your doctor
  • Stop smoking: hypnosis, acupuncture, smoking cessation patches
  • Brain training: work out your brain every day: read, computer games, crossword puzzles, take a course, start a new activity. Several observational studies have indicated that people who do cognitive stimulation activities may have a lower risk of cognitive decline and dementia. Furthermore, studies show that regular brain exercises can improve some aspects of memory and thinking particularly for middle age and older people
  • Manage diabetes: follow diet plan, maintain weight, exercise, take medications/insulin as prescribed, blood tests as ordered, learn to recognize the early signs of low/high blood sugar, and most importantly, schedule regular follow up visits with your Doctor
  • Management of midlife hypertension: limit salt intake, take prescribed medications, stress management techniques, healthy diet, exercise, regular BP checks
  • Healthy eating pattern: reduce saturated fat, limit salt and sugar e.g. Mediterranean diet
  • Management of cholesterol: take medications as prescribed, exercise, reduce fat intake
  • Limit alcohol intake: as per Canadian guidelines, men should have a maximum of 15 drinks per week, whilst women should have a maximum of 10 drinks per week
  • Keep socially engaged: meet with friends, go to social events, volunteer
  • Protect your brain: wear protective helmets when playing contact sports or an activity that may cause a head injury e.g. cycling, skiing, and follow concussion protocol if injured

Given that all these risk factors are largely correlated, it may be that living a healthy, socially engaged life is the best way to prevent dementia, and that one single factor is insufficient to prevent dementia.

Given that one in three babies born today will develop dementia in their lifetime (Lewis, 2015), it is never too young to develop good habits, but mid-life (age 40-64) is a good time to start making healthy choices if you are not already doing so.  

Dementia myths exposed

Dementia Aware: what you need to know about dementia myths and misconceptions

Advances in health care and technology has enhanced longevity, and people are healthier and living longer than ever before.

This has resulted in an increased prevalence of dementia (Alzheimer’s Disease International & WHO, 2012).

Dementia is highly stigmatized and universally feared. It is often perceived as a normal part of ageing and that people with dementia do not live a normal, happy life and will become unpredictable, aggressive and/or wander.

These misconceptions have resulted in many people not seeking diagnosis and treatment, and those already diagnosed with dementia feeling shame, fear and embarrassment, becoming socially isolated and reluctant to ask for help.

By promoting a better understanding of dementia, raising public awareness and engagement, including respect for the human rights of people with dementia, and dispelling these myths and misconceptions, we can reduce stigma and discrimination and foster greater participation, social inclusion and integration for all people with dementia.

Dementia is a normal part of ageing: False

While the number of people with dementia is growing as the world population ages, dementia is not a normal part of the ageing process.

Certainly, the prevalence of dementia increases with age, for those over 80 years old, the prevalence is higher than 20 per cent and it continues to climb to over 30 per cent in those aged about 95 years old.

However, dementia can occur in people as young as 30 years old, and many people with Down’s Syndrome develop dementia at a much younger age (approximately 55 years old).

Recently, there has also been an increased incidence of men (often professional athletes) aged 40-75 years old being diagnosed with probable CTE (chronic traumatic encephalopathy), and most people with early onset Alzheimer’s disease usually develop symptoms of the disease in their 40s and 50s.

A recent study in the U.K. identified 43,000 people under the age of 65 are living with Young Onset Dementia (YOD).

Dementia and Alzheimer’s disease are the same: False

Dementia is a syndrome due to disease of the brain, usually chronic, characterized by a progressive, global deterioration in intellect including memory, learning, orientation, language, comprehension and judgment (WHO 2016).

There are over 100 types of dementia of which Alzheimer’s disease is the most common. Other types of dementia include vascular dementia, Lewy Body dementia and frontotemporal dementia.

Because each dementia affects the brain in different ways, they produce different symptoms; however, the three most common symptoms all dementias share include: loss of memory, mood changes and communication problems.

All people with dementia will become agitated/aggressive: False

Dementia affects each person differently and each person will react to events and circumstances in his/her own way. While most people with dementia will experience some behavioural changes e.g. overreaction, hoarding, repetition, disorientation, sundowning, not everyone will become agitated and/or aggressive.

Most often, behaviours are a result of increased confusion and/or anxiety and fear due to changes in the brain, as well as frustration from a decline in the person’s ability to process information and communicate his/her needs.

Behaviour is usually an expression of an unmet need e.g. thirsty, in pain, fatigued, and it is important to try and understand why the person with dementia is behaving in a way.

All people with dementia will wander and get lost: False

While wandering is quite common amongst people with dementia, not every person with dementia wanders. Wandering can be very worrying for those concerned for the person’s safety and well being.

It is important to remember that people with dementia who are wheelchair dependent can also wander and become lost. Like behaviour, it is important to understand there is always a reason for the person wandering, including:

  • Boredom
  • Confusing night with day
  • Discomfort or pain
  • A response to a dream believing that it has happened in real life
  • A change in the environment
  • Searching for something or someone from the past e.g. going home, going to work
  • People who have been used to walking long distances, or used to walk to work
  • Excess energy
  • Forget where he/she is going and why e.g. the store and then unable to find way home

People with dementia can’t function, can’t have a quality life and can’t enjoy activities: False

People with dementia can function, can have quality of life and can enjoy activities; in fact, many people live meaningful, happy, fulfilled lives. Quality of life is possible by making some lifestyle choices:

  • Eating right
  • Keeping the brain stimulated
  • Exercising (overwhelming research suggests that any type of aerobic exercise has a significant and beneficial impact on the brain and the body)
  • Participating in development of dementia policies/programs
  • A willingness to accept support/help
  • Continuing to pursue activities that give joy
  • Maintaining a social connection with the community

As the dementia progresses and more help is required, people with dementia can still participate in activities (modified to meet one’s ability and need), they can still feel joy, laugh, smile, give and receive love, they can still be in the moment and share that moment with others.

More Dementia Aware articles

About the Author

Tracey Maxfield, RN, BSN, GNC(c), DDS, is a dementia educator, consultant and advocate with over 35 years experience working with dementia populations in the U.K. and Canada.

She has worked in a variety of heath-care settings: acute care, palliative care, community care, residential care, physicians offices and community health centres..

Maxfield is a Central Okanagan Purple Angel Dementia Ambassador.

She can be reached at [email protected].

The views expressed are strictly those of the author and not necessarily those of Castanet. Castanet does not warrant the contents.

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